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Vascular Leaking, a Pivotal and Early Pathogenetic Event in Systemic Sclerosis: Should the Door Be Closed?

Bruni, Cosimo ; Frech, Tracy ; Manetti, Mirko ; Rossi, Francesca Wanda ; Furst, Daniel E ; De Paulis, Amato ; Rivellese, Felice ; Guiducci, Serena ; Matucci-Cerinic, Marco ; Bellando-Randone, Silvia

Frontiers in immunology, 2018-09, Vol.9, p.2045-2045 [Peer Reviewed Journal]

Switzerland: Frontiers Research Foundation

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Title:
Vascular Leaking, a Pivotal and Early Pathogenetic Event in Systemic Sclerosis: Should the Door Be Closed?
Author: Bruni, Cosimo ; Frech, Tracy ; Manetti, Mirko ; Rossi, Francesca Wanda ; Furst, Daniel E ; De Paulis, Amato ; Rivellese, Felice ; Guiducci, Serena ; Matucci-Cerinic, Marco ; Bellando-Randone, Silvia
Subjects: Animals ; Biomarkers ; Capillaries - metabolism ; capillary leak ; Capillary Permeability ; Control ; Disease Susceptibility ; edema ; endothelial dysfunction ; Endothelium, Vascular - metabolism ; extravasation ; Genetic aspects ; Humans ; Immune System - cytology ; Immune System - immunology ; Immune System - metabolism ; Immunology ; Inflammation ; Lymphatic Diseases - diagnosis ; Lymphatic Diseases - etiology ; Lymphatic Diseases - metabolism ; Lymphatic Diseases - physiopathology ; Scleroderma (Disease) ; Scleroderma, Systemic - diagnosis ; Scleroderma, Systemic - etiology ; Scleroderma, Systemic - metabolism ; Scleroderma, Systemic - physiopathology ; Systemic scleroderma ; systemic sclerosis ; vasculopathy
Is Part Of: Frontiers in immunology, 2018-09, Vol.9, p.2045-2045
Notes: ObjectType-Article-2
SourceType-Scholarly Journals-1
ObjectType-Feature-3
content type line 23
ObjectType-Review-1
This article was submitted to Immunological Tolerance and Regulation, a section of the journal Frontiers in Immunology
Reviewed by: Bertrand Dunogué, Assistance Publique Hopitaux De Paris (AP-HP), France; Marie-Elise Truchetet, Université de Bordeaux, France
Edited by: Jérôme Avouac, Université Paris Descartes, France
Description: The early phase of systemic sclerosis (SSc) presents edema as one of the main features: this is clinically evident in the digital swelling (puffy fingers) as well as in the edematous skin infiltration of the early active diffuse subset. Other organs could be affected by this same disease process, such as the lung (with the appearance of ground glass opacities) and the heart (with edematous changes on cardiac magnetic resonance imaging). The genesis of tissue edema is tightly linked to pathological changes in the endothelium: various reports demonstrated the effect of transforming growth factor β, vascular endothelial growth factor and hypoxia-reperfusion damage with reactive oxygen species generation in altering vascular permeability and extravasation, in particular in SSc. This condition has an alteration in the glycocalyx thickness, reducing the protection of the vessel wall and causing non-fibrotic interstitial edema, a marker of vascular leak. Moreover, changes in the junctional adhesion molecule family and other adhesion molecules, such as ICAM and VCAM, are associated with an increased myeloid cells' extravasation in the skin and increased myofibroblasts transformation with further vascular leak and cellular migration. This mini-review examines current knowledge on determinants of vascular leak in SSc, shedding light on the role of vascular protection. This could enhance further studies in the light of drug development for early treatment, suggesting that the control of vascular leakage should be considered in the same way that vasodilation and inflammation reduction, as potential therapeutic targets.
Publisher: Switzerland: Frontiers Research Foundation
Language: English

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Vascular Leaking, a Pivotal and Early Pathogenetic Event in Systemic Sclerosis: Should the Door Be Closed?

Bruni, Cosimo ; Frech, Tracy ; Manetti, Mirko ; Rossi, Francesca Wanda ; Furst, Daniel E ; De Paulis, Amato ; Rivellese, Felice ; Guiducci, Serena ; Matucci-Cerinic, Marco ; Bellando-Randone, Silvia

Switzerland: Frontiers Research Foundation

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